1. Disease Information

1.1 What is ET? (current understanding)

ET is a clonal MPN with persistent thrombocytosis and bone marrow megakaryocytic proliferation, with clinical complications dominated by thrombosis, bleeding, and less frequently progression to myelofibrosis (MF) or acute myeloid leukemia (AML) (tefferi2025essentialthrombocythemia pages 1-2, tefferi2024essentialthrombocythemia2024 pages 1-2). - A contemporary definition from the AJH 2024 update describes: “Essential thrombocythemia is a Janus kinase 2 (JAK2) mutation‑prevalent myeloproliferative neoplasm characterized by clonal thrombocytosis” (tefferi2024essentialthrombocythemia2024 pages 1-2). - The JAMA review describes ET as a “clonal myeloproliferative neoplasm characterized by persistent thrombocytosis (platelet count ≥450 × 10^9/L) and increased risks of thrombosis and bleeding” (tefferi2025essentialthrombocythemia pages 1-2).

1.2 Key identifiers and synonyms

The retrieved evidence did not contain explicit ICD-10/ICD-11/MeSH/OMIM/Orphanet/MONDO codes; however, it consistently frames ET as a Philadelphia-negative/BCR-ABL1–negative MPN with diagnostic platelet threshold ≥450 ×10^9/L (tefferi2025essentialthrombocythemia pages 1-2, tefferi2024essentialthrombocythemia2024 pages 1-2).

Table: This table summarizes the disease name, nomenclature, and identifier fields for Essential Thrombocythemia using only retrieved evidence. It is useful for knowledge-base curation because it separates supported definitional features from identifier systems whose exact codes were not captured in the current evidence set.

1.3 Evidence sources (individual vs aggregated)

Most information in this report is derived from aggregated disease-level resources (large cohorts, systematic clinical reviews, and classification updates), including 1,000-patient institutional cohorts and guideline-style reviews (loscocco2024onethousandpatients pages 1-2, gangat2024onethousandpatients pages 1-2, tefferi2024essentialthrombocythemia2024 pages 1-2).

2. Etiology

2.1 Disease causal factors (primary causes)

ET is primarily caused by somatic (acquired) driver mutations in JAK2, CALR, or MPL, which upregulate JAK–STAT signaling and promote clonal megakaryopoiesis/platelet production (tefferi2024essentialthrombocythemia2024 pages 2-2, ferrer‐marin2024essentialthrombocythaemiaa pages 1-2). - ET driver mutations are typically mutually exclusive (JAK2 vs CALR vs MPL) and present in ~80–90% of patients depending on the cohort/definition (tefferi2024essentialthrombocythemia2024 pages 1-2, tefferi2025essentialthrombocythemia pages 1-2).

2.2 Risk factors

Host/clinical risk factors that increase thrombotic risk include: prior thrombosis, age >60 years, JAK2 mutation, and cardiovascular risk factors (e.g., hypertension) (tefferi2025essentialthrombocythemia pages 1-2, tefferi2024essentialthrombocythemia2024 pages 1-2). - In a Mayo 1,000-patient cohort (1967–2023), multivariable predictors of outcomes included hypertension (HR ~1.7 for overall survival; HR ~1.7 for arterial thrombosis-free survival) and JAK2 mutation (HR ~1.8 for arterial thrombosis-free survival) (gangat2024onethousandpatients pages 1-2).

Genetic risk/prognostic modifiers (co-mutations): additional somatic mutations are common and influence prognosis. - AJH 2024 update: ~50% have additional mutations (e.g., TET2, ASXL1, DNMT3A, SF3B1), and mutation associations include JAK2V617F with thrombosis and MPL/CALR-1 with increased MF transformation risk (tefferi2024essentialthrombocythemia2024 pages 1-2). - The 2024 BJH review notes that “triple-negative” ET may harbor other myeloid mutations including ASXL1, DNMT3A, TET2, EZH2, IDH1/2, RUNX1, SRSF2, SF3B1, TP53 (ferrer‐marin2024essentialthrombocythaemiaa pages 1-2).

2.3 Protective factors

No explicit protective genetic variants or environmental protective factors were captured in the retrieved evidence. However, both large-cohort analyses and expert updates suggest aspirin therapy is associated with reduced thrombosis risk in ET populations (a preventive/mitigating factor rather than primary prevention) (gangat2024onethousandpatients pages 1-2).

2.4 Gene–environment interactions

Direct gene–environment interaction evidence was not captured in the retrieved sources. Clinically, mutation status and cardiovascular risk factors interact in determining thrombotic risk and antiplatelet strategy (tefferi2025essentialthrombocythemia pages 1-2, ferrer‐marin2024essentialthrombocythaemiaa pages 4-5).

3. Phenotypes

3.1 Core clinical phenotypes (with suggested HPO terms)

ET phenotypes span symptoms, signs, and lab/pathology abnormalities (tefferi2025essentialthrombocythemia pages 1-2, tefferi2024essentialthrombocythemia2024 pages 1-2).

A. Laboratory / hematologic - Persistent thrombocytosis (platelets ≥450 ×10^9/L) (diagnostic threshold) (tefferi2025essentialthrombocythemia pages 1-2, tefferi2024essentialthrombocythemia2024 pages 4-5). - Suggested HPO: Thrombocytosis (HP:0001894).

B. Thrombotic manifestations - Increased risk of arterial thrombosis and venous thrombosis (tefferi2025essentialthrombocythemia pages 1-2). - JAMA review: increased risk of arterial thrombosis 11% and venous thrombosis 7% (tefferi2025essentialthrombocythemia pages 1-2). - Suggested HPO: Thrombosis (HP:0001977); Arterial thrombosis (HP:0031048); Venous thrombosis (HP:0004936).

C. Bleeding manifestations - Hemorrhagic complications reported (JAMA review: 8%) (tefferi2025essentialthrombocythemia pages 1-2). - Bleeding risk can be associated with acquired von Willebrand factor abnormalities, especially with extreme thrombocytosis (venkat2024riskofbleeding pages 3-4, tefferi2024essentialthrombocythemia2024 pages 11-11). - Suggested HPO: Abnormal bleeding (HP:0001892).

D. Microcirculatory symptoms - Microcirculatory symptoms such as “headaches, lightheadedness, and acral paresthesias” are noted (tefferi2024essentialthrombocythemia2024 pages 1-2). - Suggested HPO: Headache (HP:0002315); Lightheadedness (HP:0030931); Paresthesia (HP:0003401).

E. Disease evolution - Progression to myelofibrosis and AML occurs in a minority (tefferi2025essentialthrombocythemia pages 1-2, tefferi2024essentialthrombocythemia2024 pages 1-2). - Suggested HPO: Myelofibrosis (HP:0005532); Acute myeloid leukemia (HP:0004808).

3.2 Frequency and progression (selected recent statistics)

• JAMA review: at median 8.5 years from diagnosis, ~10% develop MF and ~3% AML (tefferi2025essentialthrombocythemia pages 1-2).
• Review estimate: MF at 15 years 4–11% and AML 2–5% (lazar2024diagnosisandmanagement pages 1-3).

3.3 Quality of life impact

Formal QoL instrument results were not captured directly, but symptom burden is a recurring treatment target; ruxolitinib is noted to be superior mainly for symptom control in hydroxyurea-resistant/intolerant ET in MAJIC-ET-related summaries (gunawan2018ruxolitinibforthe pages 1-2, ferrer‐marin2024essentialthrombocythaemiaa pages 7-8).

4. Genetic / Molecular Information

4.1 Causal genes (driver genes)

• JAK2, CALR, MPL are canonical ET driver genes that activate JAK–STAT signaling (tefferi2024essentialthrombocythemia2024 pages 1-2, tefferi2024essentialthrombocythemia2024 pages 2-2).

4.2 Driver mutation frequencies (recent large cohorts)

Large 2024 real-world cohorts provide high-confidence mutation frequency estimates: - Mayo cohort (n=1000): JAK2/CALR/MPL 62%/27%/3%, triple-negative 8% (gangat2024onethousandpatients pages 1-2). - Florence-CRIMM cohort (n=1000): JAK2/CALR/MPL 66%/19%/4%, triple-negative 11% (loscocco2024onethousandpatients pages 1-2). - JAMA review: approximately 90% have JAK–STAT-activating variants: JAK2 64%, CALR 23%, MPL 4% (tefferi2025essentialthrombocythemia pages 1-2).

Table: This table compiles key quantitative epidemiology, mutation frequency, thrombosis/bleeding risk, transformation, and survival data for essential thrombocythemia from the retrieved evidence. It is useful for quickly comparing population-level estimates with large real-world Mayo and Florence 1000-patient cohorts.

4.3 Pathogenic variant classes (high-level)

• JAK2V617F is a gain-of-function driver in ET and is “JAK2 mutation-prevalent” in ET (tefferi2024essentialthrombocythemia2024 pages 2-2, tefferi2024essentialthrombocythemia2024 pages 1-2).
• CALR mutations in ET are typically frameshift variants in exon 9 (type 1/type 2) that activate MPL/JAK–STAT (tefferi2024essentialthrombocythemia2024 pages 2-2).
• MPL mutations include W515 variants and other activating lesions (tefferi2024essentialthrombocythemia2024 pages 2-2).

4.4 Modifier/co-mutations and prognostic implications

• The AJH 2024 update reports frequent additional mutations (e.g., TET2 9–11%, ASXL1 7–20%, DNMT3A 7%, SF3B1 5%) and links certain lesions to clinical trajectories (e.g., TP53 with leukemic transformation; spliceosome mutations with inferior survival; MPL/CALR-1 with MF transformation risk) (tefferi2024essentialthrombocythemia2024 pages 1-2).

4.5 Epigenetic and chromosomal abnormalities

• Abnormal karyotype appears in <10% of patients in the AJH 2024 update (tefferi2024essentialthrombocythemia2024 pages 1-2), and 6% in the Mayo 1,000 cohort (gangat2024onethousandpatients pages 1-2).

5. Environmental Information

No specific environmental toxins, lifestyle exposures, or infectious triggers were captured as causal contributors in the retrieved ET-focused evidence. ET risk/complications are clinically influenced by cardiovascular risk factors (e.g., hypertension, diabetes), but these are generally modeled as modifiers of thrombotic risk rather than etiologic exposures (tefferi2025essentialthrombocythemia pages 1-2, gangat2024onethousandpatients pages 1-2).

6. Mechanism / Pathophysiology

6.1 Core causal chain (driver mutation → signaling → megakaryopoiesis → clinical events)

1. Somatic driver mutation in JAK2/CALR/MPL occurs in a hematopoietic stem/progenitor clone (clonal hematopoiesis) (tefferi2024essentialthrombocythemia2024 pages 2-2).
2. Driver mutations activate JAK–STAT signaling and increase megakaryocyte/platelet production.
3. JAMA review: driver variants “upregulate the JAK-STAT” pathway and “bypass[] the need for growth factor ligands… which stimulate myeloproliferation and megakaryocyte production” (tefferi2025essentialthrombocythemia pages 1-2).
4. AJH 2024 update: JAK2V617F exerts “a primary effect on platelet production” and “development of the ET phenotype” (tefferi2024essentialthrombocythemia2024 pages 2-2).
5. Clinical manifestations arise from:
6. Thrombosis (arterial/venous), influenced by mutation status (especially JAK2), age, and cardiovascular risk factors (tefferi2025essentialthrombocythemia pages 1-2, tefferi2024essentialthrombocythemia2024 pages 1-2).
7. Bleeding, particularly when extreme thrombocytosis is associated with acquired von Willebrand factor defects (venkat2024riskofbleeding pages 3-4).
8. Long-term clonal evolution with additional mutations leading to MF/AML transformation in a minority (tefferi2025essentialthrombocythemia pages 1-2, tefferi2024essentialthrombocythemia2024 pages 1-2).

6.2 Mechanistic notes: CALR and MPL

• AJH 2024 update summarizes a mechanism for mutant CALR: mutant CALR can bind MPL, promote MPL dimerization/trafficking, and activate JAK–STAT (tefferi2024essentialthrombocythemia2024 pages 2-2).

6.3 Inflammation and non-driver mutations

• AJH 2024 update notes “co-existence of an inflammatory state with aberrant cytokine expression” and that phenotype is modified by “other co-occurring mutations, including those of epigenetic regulators and their order of acquisition” (tefferi2024essentialthrombocythemia2024 pages 2-2).
• It also cautions that “activated JAK–STAT is a non-specific phenomenon in cancer,” and that JAK inhibitors have not selectively suppressed the ET clone in general, reflecting biologic complexity (tefferi2024essentialthrombocythemia2024 pages 2-2).

6.4 Suggested pathway / ontology terms

• GO (biological process): JAK-STAT cascade (e.g., “JAK-STAT signaling pathway”); “megakaryocyte differentiation”; “platelet production”; “inflammatory response”. (Ontology suggestions; not directly asserted by retrieved evidence.)
• CL (cell types): hematopoietic stem cell; megakaryocyte; megakaryocyte progenitor. (Ontology suggestions.)

7. Anatomical Structures Affected

7.1 Primary

• Bone marrow: characteristic megakaryocytic proliferation and clustering on biopsy (tefferi2024essentialthrombocythemia2024 pages 4-5, loscocco2024onethousandpatients pages 1-2).

7.2 Secondary (complication targets)

• Vascular beds and organs affected by thrombosis/bleeding (brain, coronary circulation, venous system) are clinically relevant (stroke/thrombotic events are referenced as manifestations) (lazar2024diagnosisandmanagement pages 1-3, tefferi2025essentialthrombocythemia pages 1-2).

7.3 Suggested anatomy ontology terms

• UBERON: bone marrow; blood; vasculature. (Ontology suggestions.)

8. Temporal Development

• Onset: typically adult; median age at diagnosis ~59 years (tefferi2025essentialthrombocythemia pages 1-2).
• Course: often indolent, interrupted by thrombotic/hemorrhagic events; transformation to MF/AML occurs in a minority (tefferi2024essentialthrombocythemia2024 pages 1-2).
• Progression statistics: JAMA review reports ~10% MF and ~3% AML at median 8.5 years (tefferi2025essentialthrombocythemia pages 1-2).

9. Inheritance and Population

9.1 Inheritance

ET is principally a somatic clonal hematopoietic disease driven by acquired mutations in hematopoietic stem/progenitor cells; a Mendelian inheritance pattern was not supported by the retrieved evidence (tefferi2024essentialthrombocythemia2024 pages 2-2, tefferi2024essentialthrombocythemia2024 pages 1-2).

9.2 Epidemiology (recent statistics)

• Annual incidence estimates:
• US annual incidence 1.5/100,000 (JAMA review) (tefferi2025essentialthrombocythemia pages 1-2).
• Review estimate 0.6–2.5/100,000/year (BJH 2024 review) (ferrer‐marin2024essentialthrombocythaemiaa pages 1-2).
• Prevalence estimate (review): 38–57/100,000 (lazar2024diagnosisandmanagement pages 1-3).
• Sex distribution: ~63–65% female in two large 2024 cohorts (gangat2024onethousandpatients pages 1-2, loscocco2024onethousandpatients pages 1-2).

10. Diagnostics

10.1 Diagnostic criteria (WHO/ICC-aligned core elements)

The AJH 2024 update reproduces the ICC framework: diagnosis requires “meeting all four major criteria or meeting the first three major criteria plus one minor criterion” (tefferi2024essentialthrombocythemia2024 pages 4-5). Major criteria elements include: 1. Platelets ≥450 ×10^9/L (tefferi2024essentialthrombocythemia2024 pages 4-5). 2. Bone marrow: megakaryocyte proliferation with mature cytology/hyperlobulated nuclei, loose clustering, and absent or ≤ grade 1 fibrosis (tefferi2024essentialthrombocythemia2024 pages 4-5). 3. Exclusion of other myeloid neoplasms (including PV, prefibrotic MF, CML) (tefferi2024essentialthrombocythemia2024 pages 4-5). 4. Presence of driver mutation JAK2/CALR/MPL (tefferi2024essentialthrombocythemia2024 pages 4-5).

Bone marrow morphology emphasized in practice includes normocellularity for age, increased mature megakaryocytes in loose clusters, and reticulin fibrosis < grade 1 (loscocco2024onethousandpatients pages 1-2).

10.2 Differential diagnosis (key exclusions)

Differential diagnoses include other MPNs and reactive thrombocytosis: - JAMA review lists PV, primary myelofibrosis, CML, inflammatory conditions, infections, splenectomy, iron deficiency anemia, and solid tumors among differentials (tefferi2025essentialthrombocythemia pages 1-2).

10.3 Recommended molecular testing

WHO/ICC-aligned approaches emphasize driver mutation testing for JAK2/CALR/MPL (thiele2025evolutionofwho pages 3-4, tefferi2024essentialthrombocythemia2024 pages 4-5).

10.4 Suggested diagnostic ontology terms

• LOINC/measurement: platelet count; vWF activity/antigen when assessing bleeding/AvWS (venkat2024riskofbleeding pages 3-4).

11. Outcome / Prognosis

11.1 Survival and transformation

• AJH 2024 update: median survival ~18 years, and notes survival “>>35 years in younger patients” (tefferi2024essentialthrombocythemia2024 pages 1-2).
• JAMA review: “The median overall survival exceeds 35 years in those diagnosed at 40 years or younger” (tefferi2025essentialthrombocythemia pages 1-2).
• JAMA review: at median 8.5 years, ~10% MF and ~3% AML (tefferi2025essentialthrombocythemia pages 1-2).
• AJH 2024 update: leukemic transformation at 10 years is <1% overall (tefferi2024essentialthrombocythemia2024 pages 1-2).

11.2 Thrombosis and bleeding burden (recently summarized)

• JAMA review reports ET patients are at increased risk of arterial thrombosis (11%), venous thrombosis (7%), and hemorrhagic complications (8%) (tefferi2025essentialthrombocythemia pages 1-2).
• Cohort-based risk models in Mayo 1,000-patient series delineated 20-year leukemia/myelofibrosis incidences ranging 3%/21% to 12.8%/49% across risk groups (gangat2024onethousandpatients pages 1-2).

12. Treatment

12.1 Treatment goals and strategy (risk-adapted, real-world)

The AJH 2024 update frames ET therapy around thrombosis prevention, using risk groups incorporating thrombosis history, age, and JAK2 status (tefferi2024essentialthrombocythemia2024 pages 1-2).

Figure-based algorithm (visual evidence) - The AJH 2024 update provides a treatment algorithm stratifying “very low / low / intermediate / high” risk and selecting observation vs aspirin vs cytoreduction (tefferi2024essentialthrombocythemia2024 media 9b23e152).

12.2 Antiplatelet therapy (aspirin)

• AJH 2024 update: aspirin is a baseline therapy and should be monitored closely when extreme thrombocytosis/AvWS is present (tefferi2024essentialthrombocythemia2024 pages 11-11, tefferi2024essentialthrombocythemia2024 pages 10-11).
• Aspirin dosing considerations: increased platelet turnover may lead to incomplete inhibition with once-daily dosing; AJH 2024 reports BID/TID regimens reduce platelet activation more than QD (tefferi2024essentialthrombocythemia2024 pages 11-12).
• In the Mayo 1,000 cohort, aspirin therapy “appeared to mitigate both arterial (HR 0.4) and venous (HR 0.4) thrombosis risk” (gangat2024onethousandpatients pages 1-2).

MAXO suggestion: antiplatelet therapy.

12.3 Cytoreductive therapy (standard of care in high-risk ET)

• AJH 2024 update: hydroxyurea is current first-line cytoreduction in high-risk ET (tefferi2024essentialthrombocythemia2024 pages 10-11).
• Second-line: “pegylated IFN‑α or busulfan” in hydroxyurea-intolerant/refractory patients (tefferi2024essentialthrombocythemia2024 pages 11-11).
• AJH 2024 update reports randomized comparisons with anagrelide, noting different profiles: hydroxyurea reduced arterial thrombosis/major bleeding/fibrotic progression, while anagrelide was better at preventing venous thrombosis but had higher adverse-event dropout (tefferi2024essentialthrombocythemia2024 pages 12-13).

MAXO suggestions: cytoreductive therapy; interferon therapy.

12.4 Managing extreme thrombocytosis and bleeding risk (2024 evidence update)

A key 2024 development is more granular evidence on bleeding in ET with extreme thrombocytosis (ExT). - Venkat et al. (Blood Adv. Dec 2024) concludes: “There is no clear indication for cytoreduction to decrease bleeding risk based on a platelet threshold of 1 million alone” (abstract key points) (venkat2024riskofbleeding pages 1-2). - The same study reports ExT is associated with lower vWF antigen/activity, consistent with acquired vWF abnormalities (venkat2024riskofbleeding pages 3-4). - AJH 2024 emphasizes that platelet extremes alone do not necessarily increase thrombosis/hemorrhage risk, and that therapy modification is mainly warranted when ExT is accompanied by AvWS (tefferi2024essentialthrombocythemia2024 pages 11-12).

12.5 Targeted and emerging therapies (2023–2024 landscape)

Ruxolitinib (JAK1/2 inhibitor; selected settings) - 2024 BJH review summarizes that in hydroxyurea-resistant/intolerant ET, ruxolitinib improved some symptoms but did not reduce thrombosis/bleeding/transformation and did not show superiority over best available therapy (MAJIC-ET) (ferrer‐marin2024essentialthrombocythaemiaa pages 7-8). - Earlier ET-specific review similarly notes superiority mainly in symptom control vs conventional therapy (gunawan2018ruxolitinibforthe pages 1-2).

Bomedemstat (LSD1 inhibitor; emerging therapy) - AJH 2024 update: bomedemstat is “an orally active LSD1 inhibitor” and in a phase 2 study (NCT04254978) achieved platelet response (≤400×10^9/L) in 94% with median time to response 8 weeks; common AEs included dysgeusia (43%), constipation (27%), fatigue (23%), thrombocytopenia (23%), and discontinuation ~20% (tefferi2024essentialthrombocythemia2024 pages 17-17). - Phase 3 registry trial: NCT06079879 compares bomedemstat vs best available therapy in HU inadequate response/intolerant ET; start date 2023‑12‑31; planned enrollment 340; primary endpoint durable clinicohematologic response by week 52 (NCT06079879 chunk 1).

Ropeginterferon alfa-2b (phase 3 trial in ET) - SURPASS-ET registry trial: NCT04285086 is an open-label, randomized, phase 3 study comparing ropeginterferon alfa‑2b vs anagrelide in HU-resistant/intolerant ET; enrollment 174; primary outcomes include blood count remission, symptom improvement, and absence of thrombotic/hemorrhagic events (NCT04285086 chunk 1).

MAXO suggestions: interferon therapy; JAK inhibitor therapy; histone demethylase inhibitor therapy.

13. Prevention

No primary-prevention interventions were captured (ET is primarily somatic clonal disease). Secondary/tertiary prevention focuses on prevention of thrombosis/bleeding complications via risk-adapted antiplatelet therapy and cytoreduction (tefferi2024essentialthrombocythemia2024 pages 1-2, ferrer‐marin2024essentialthrombocythaemiaa pages 3-4).

14. Other Species / Natural Disease

No naturally occurring animal disease evidence for ET was captured in the retrieved sources for this report.

15. Model Organisms

While mechanistic statements reference preclinical support for mutant CALR/MPL biology, specific model organism systems and phenotypes were not captured in the retrieved evidence excerpts (tefferi2024essentialthrombocythemia2024 pages 2-2).

Notes on evidence gaps and curation constraints

• Ontology codes (MONDO/MeSH/ICD/OMIM/Orphanet) were not present in the retrieved excerpts, so this report explicitly flags them as “not captured in retrieved sources” rather than guessing (artifact-00).
• PMIDs were not provided in the retrieved text snippets; therefore, the report cites DOI/URL-bearing sources as retrieved by the tool. Where the user requires PMIDs, additional database-specific retrieval would be needed.

Visual evidence (treatment algorithm)

• ET treatment algorithm figure from the AJH 2024 update: (tefferi2024essentialthrombocythemia2024 media 9b23e152).

References

1. (loscocco2024onethousandpatients pages 1-2): Giuseppe G. Loscocco, Francesca Gesullo, Giulio Capecchi, Alessandro Atanasio, Chiara Maccari, Francesco Mannelli, Alessandro M. Vannucchi, and Paola Guglielmelli. One thousand patients with essential thrombocythemia: the florence-crimm experience. Blood Cancer Journal, Jan 2024. URL: https://doi.org/10.1038/s41408-023-00968-7, doi:10.1038/s41408-023-00968-7. This article has 43 citations and is from a domain leading peer-reviewed journal.

2. (ferrer‐marin2024essentialthrombocythaemiaa pages 1-2): Francisca Ferrer‐Marín, Juan Carlos Hernández‐Boluda, and Alberto Alvarez‐Larrán. Essential thrombocythaemia: a contemporary approach with new drugs on the horizon. British Journal of Haematology, 204:1605-1616, Apr 2024. URL: https://doi.org/10.1111/bjh.19403, doi:10.1111/bjh.19403. This article has 7 citations and is from a domain leading peer-reviewed journal.

3. (tefferi2024essentialthrombocythemia2024 pages 1-2): Ayalew Tefferi, Alessandro Maria Vannucchi, and Tiziano Barbui. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. American Journal of Hematology, 99:697-718, Jan 2024. URL: https://doi.org/10.1002/ajh.27216, doi:10.1002/ajh.27216. This article has 141 citations and is from a domain leading peer-reviewed journal.

4. (tefferi2025essentialthrombocythemia pages 1-2): Ayalew Tefferi, Naseema Gangat, Giuseppe Gaetano Loscocco, Paola Guglielmelli, Natasha Szuber, Animesh Pardanani, Attilio Orazi, Tiziano Barbui, and Alessandro Maria Vannucchi. Essential thrombocythemia. JAMA, 333:701, Feb 2025. URL: https://doi.org/10.1001/jama.2024.25349, doi:10.1001/jama.2024.25349. This article has 22 citations.

5. (gangat2024onethousandpatients pages 1-2): Naseema Gangat, Omer Karrar, Aref Al-Kali, Kebede H. Begna, Michelle A. Elliott, Alexandra P. Wolanskyj-Spinner, Animesh Pardanani, Curtis A. Hanson, Rhett P. Ketterling, and Ayalew Tefferi. One thousand patients with essential thrombocythemia: the mayo clinic experience. Blood Cancer Journal, Jan 2024. URL: https://doi.org/10.1038/s41408-023-00972-x, doi:10.1038/s41408-023-00972-x. This article has 57 citations and is from a domain leading peer-reviewed journal.

6. (venkat2024riskofbleeding pages 1-2): Rathnam K. Venkat, Robert A. Redd, Amyah C. Harris, Martin J. Aryee, Anna E. Marneth, Baransel Kamaz, Chulwoo J. Kim, Mohammed Wazir, Lachelle D. Weeks, Maximilian Stahl, Daniel J. DeAngelo, R. Coleman Lindsley, Marlise R. Luskin, Gabriela S. Hobbs, and Joan How. Risk of bleeding in patients with essential thrombocythemia and extreme thrombocytosis. Blood Advances, 8:6043-6054, Dec 2024. URL: https://doi.org/10.1182/bloodadvances.2024013777, doi:10.1182/bloodadvances.2024013777. This article has 10 citations and is from a peer-reviewed journal.

7. (tefferi2024essentialthrombocythemia2024 pages 4-5): Ayalew Tefferi, Alessandro Maria Vannucchi, and Tiziano Barbui. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. American Journal of Hematology, 99:697-718, Jan 2024. URL: https://doi.org/10.1002/ajh.27216, doi:10.1002/ajh.27216. This article has 141 citations and is from a domain leading peer-reviewed journal.

8. (thiele2025evolutionofwho pages 3-3): Jürgen Thiele, Hans Michael Kvasnicka, Umberto Gianelli, Daniel A. Arber, Ayalew Tefferi, Alessandro M. Vannucchi, Tiziano Barbui, and Attilio Orazi. Evolution of who diagnostic criteria in “classical myeloproliferative neoplasms” compared with the international consensus classification. Blood Cancer Journal, Mar 2025. URL: https://doi.org/10.1038/s41408-025-01235-7, doi:10.1038/s41408-025-01235-7. This article has 9 citations and is from a domain leading peer-reviewed journal.

9. (allen2022thrombocytosisandessential pages 5-6): Julie Allen and Tracy Stokol. Thrombocytosis and essential thrombocythemia. Schalm's Veterinary Hematology, pages 721-730, Mar 2022. URL: https://doi.org/10.1002/9781119500537.ch82, doi:10.1002/9781119500537.ch82. This article has 7 citations.

10. (tefferi2024essentialthrombocythemia2024 pages 2-2): Ayalew Tefferi, Alessandro Maria Vannucchi, and Tiziano Barbui. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. American Journal of Hematology, 99:697-718, Jan 2024. URL: https://doi.org/10.1002/ajh.27216, doi:10.1002/ajh.27216. This article has 141 citations and is from a domain leading peer-reviewed journal.

11. (ferrer‐marin2024essentialthrombocythaemiaa pages 4-5): Francisca Ferrer‐Marín, Juan Carlos Hernández‐Boluda, and Alberto Alvarez‐Larrán. Essential thrombocythaemia: a contemporary approach with new drugs on the horizon. British Journal of Haematology, 204:1605-1616, Apr 2024. URL: https://doi.org/10.1111/bjh.19403, doi:10.1111/bjh.19403. This article has 7 citations and is from a domain leading peer-reviewed journal.

12. (venkat2024riskofbleeding pages 3-4): Rathnam K. Venkat, Robert A. Redd, Amyah C. Harris, Martin J. Aryee, Anna E. Marneth, Baransel Kamaz, Chulwoo J. Kim, Mohammed Wazir, Lachelle D. Weeks, Maximilian Stahl, Daniel J. DeAngelo, R. Coleman Lindsley, Marlise R. Luskin, Gabriela S. Hobbs, and Joan How. Risk of bleeding in patients with essential thrombocythemia and extreme thrombocytosis. Blood Advances, 8:6043-6054, Dec 2024. URL: https://doi.org/10.1182/bloodadvances.2024013777, doi:10.1182/bloodadvances.2024013777. This article has 10 citations and is from a peer-reviewed journal.

13. (tefferi2024essentialthrombocythemia2024 pages 11-11): Ayalew Tefferi, Alessandro Maria Vannucchi, and Tiziano Barbui. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. American Journal of Hematology, 99:697-718, Jan 2024. URL: https://doi.org/10.1002/ajh.27216, doi:10.1002/ajh.27216. This article has 141 citations and is from a domain leading peer-reviewed journal.

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16. (ferrer‐marin2024essentialthrombocythaemiaa pages 7-8): Francisca Ferrer‐Marín, Juan Carlos Hernández‐Boluda, and Alberto Alvarez‐Larrán. Essential thrombocythaemia: a contemporary approach with new drugs on the horizon. British Journal of Haematology, 204:1605-1616, Apr 2024. URL: https://doi.org/10.1111/bjh.19403, doi:10.1111/bjh.19403. This article has 7 citations and is from a domain leading peer-reviewed journal.

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18. (thiele2025evolutionofwho pages 3-4): Jürgen Thiele, Hans Michael Kvasnicka, Umberto Gianelli, Daniel A. Arber, Ayalew Tefferi, Alessandro M. Vannucchi, Tiziano Barbui, and Attilio Orazi. Evolution of who diagnostic criteria in “classical myeloproliferative neoplasms” compared with the international consensus classification. Blood Cancer Journal, Mar 2025. URL: https://doi.org/10.1038/s41408-025-01235-7, doi:10.1038/s41408-025-01235-7. This article has 9 citations and is from a domain leading peer-reviewed journal.

19. (tefferi2024essentialthrombocythemia2024 media 9b23e152): Ayalew Tefferi, Alessandro Maria Vannucchi, and Tiziano Barbui. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. American Journal of Hematology, 99:697-718, Jan 2024. URL: https://doi.org/10.1002/ajh.27216, doi:10.1002/ajh.27216. This article has 141 citations and is from a domain leading peer-reviewed journal.

20. (tefferi2024essentialthrombocythemia2024 pages 10-11): Ayalew Tefferi, Alessandro Maria Vannucchi, and Tiziano Barbui. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. American Journal of Hematology, 99:697-718, Jan 2024. URL: https://doi.org/10.1002/ajh.27216, doi:10.1002/ajh.27216. This article has 141 citations and is from a domain leading peer-reviewed journal.

21. (tefferi2024essentialthrombocythemia2024 pages 11-12): Ayalew Tefferi, Alessandro Maria Vannucchi, and Tiziano Barbui. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. American Journal of Hematology, 99:697-718, Jan 2024. URL: https://doi.org/10.1002/ajh.27216, doi:10.1002/ajh.27216. This article has 141 citations and is from a domain leading peer-reviewed journal.

22. (tefferi2024essentialthrombocythemia2024 pages 12-13): Ayalew Tefferi, Alessandro Maria Vannucchi, and Tiziano Barbui. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. American Journal of Hematology, 99:697-718, Jan 2024. URL: https://doi.org/10.1002/ajh.27216, doi:10.1002/ajh.27216. This article has 141 citations and is from a domain leading peer-reviewed journal.

23. (tefferi2024essentialthrombocythemia2024 pages 17-17): Ayalew Tefferi, Alessandro Maria Vannucchi, and Tiziano Barbui. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. American Journal of Hematology, 99:697-718, Jan 2024. URL: https://doi.org/10.1002/ajh.27216, doi:10.1002/ajh.27216. This article has 141 citations and is from a domain leading peer-reviewed journal.

24. (NCT06079879 chunk 1): A Study of Bomedemstat (IMG-7289/MK-3543) Compared to Best Available Therapy (BAT) in Participants With Essential Thrombocythemia and an Inadequate Response or Intolerance of Hydroxyurea (MK-3543-006). Merck Sharp & Dohme LLC. 2023. ClinicalTrials.gov Identifier: NCT06079879

25. (NCT04285086 chunk 1): Ropeginterferon Alfa-2b (P1101) vs. Anagrelide in Essential Thrombocythemia Patients With Hydroxyurea Resistance or Intolerance. PharmaEssentia. 2020. ClinicalTrials.gov Identifier: NCT04285086

26. (ferrer‐marin2024essentialthrombocythaemiaa pages 3-4): Francisca Ferrer‐Marín, Juan Carlos Hernández‐Boluda, and Alberto Alvarez‐Larrán. Essential thrombocythaemia: a contemporary approach with new drugs on the horizon. British Journal of Haematology, 204:1605-1616, Apr 2024. URL: https://doi.org/10.1111/bjh.19403, doi:10.1111/bjh.19403. This article has 7 citations and is from a domain leading peer-reviewed journal.